Surgical Hepatobiliary Diseases

Case 1 Surgical Hepatobiliary Diseases M. Sinanan MD, PhD 48 yo WF with RUQ, postprandial pain, fever, nausea, vomiting, and tenderness. US and HIDA done Issues 1. 2. Interpretation Adequate WU? 2 Normal Studies Case 1 48 yo WF with RUQ, postprandial pain, tenderness, and US and HIDA done Issues Interpretation? Ultrasound Gallbladder with gallstone dz 3 Case 1 Gallstone Disease I 48 yo WF with RUQ, postprandial pain, nausea, vomiting, fever, and tenderness. US + for GB wall edema, pericholecystic fluid, no gallstones seen HIDA + for cystic duct obstruction Dx: Acute cholecystitis. Cause? 2. 3. 4. 5. 6. 7. Adequate WU? Indication for surgery? Options for management Risks of surgery Consent Technical issues in procedure Consequences of treatment Prevalence: 20% US population (52M in US) Female, middle aged, postpartum, + FH Issues 1. 4 5 NA population increased prevalence 90% cholesterol, 10% pigment in character 50% symptomatic, 20% over 20 yr rate of becoming symptomatic Symptoms tend to be similar to presentation Once gallstones are present, they persist Once symptomatic, always symptomatic Complications of gallstone disease: acute cholecystitis, CBD obstruction, pancreatitis, cholecystoduodenal fistula, GB cancer 6

Gallstone Disease II History LFT s US Bili <3, elevated alk phos, normal ALT/AST CBD railroad tracks Gallstones GB size, thickness of wall, pericholecystic fluid CBD diameter Indicates function of GB, tracer uptake, biliary tract anatomy, drainage Non-filling GB acute cholecystitis EF <40% with CCK chronic cholecystitis Rx Gallstones with acustic shadow HIDA scan US Images of Gallstone Disease Diagnosis Cholecystectomy (laparoscopic, open) Ursodeoxycholic acid Actigall (1-3 gallstones, < 1 cm diameter, noncalcified) Lithotripsy Cholecystostomy (acute cholecystitis in surgically unfit patient) Consequences of cholecystectomy 50% have looser BM s initially, will correct for most but 3-5% suffer long term with diarrhea Bile leak, biliary tract injury 0.3% 7 Laparoscopic Cholangiogram: Normal Anatomy 8 Images: Lap Chole for Acute Cholecystitis Steps in procedure 1 2 4 5 3 6 9 10 Case 1: Outcome Lap chole for Acute cholecystitis Variations in Biliary Anatomy Acute, necrotizing cholecystitis 11 Bile Duct Variation Hepatic Artery Variation 12

Choledocholithiasis p 95% of CBD stones from GB p Untreated, 80% chance of complications: biliary obstruction, cholangitis, pancreatitis p Management: always resolve 1. Endoscopic ERCP 2. Laparoscopic/open CBD exploration during chole 3. Transhepatic cholangiography - PTC p Primary CBD stones pigment n Associated with biliary obstruction, stasis, foreign body n Oriental cholangiohepatitis At laparoscopy At ERCP Case 2 p 61 yo BM with 5 kg wt loss, dark urine, clay-colored stools but no pain. Afebrile. Jaundice, mild RUQ tenderness and fullness on clinical exam. No other physical findings. n Labs p LFT s: T. Bili 8, Alk Phos 680, ALT 140, AST 160, amylase 27. n Next step? 13 14 US Normal liver Case 2: Studies p Hepatitis serology p US 15 16 Case 2: Studies Normal ERCP p US p CT Scan 17 18

Case 2: Studies Normal MRI Biliary Tract p US p CT p ERCP 19 20 Studies p US p CT p ERCP p MRCP Studies p US p CT p ERCP p MRCP n vs adjacent vessels 21 22 Case 2 p 61 yo WM with wt loss, dark urine, claycolored stools but no pain n LFT Bili 8, Alk Phos 600, ALT 80, AST 45 p Issues n DDx n Evaluation n Radiographic studies n Curative vs noncurative n Natural history of each treatment option n Surgical options 23 Painless jaundice: DDx p Indirect bili elevation, no ductal dilation n Hepatitis liver failure (usually associated symptoms) p Direct bili elevation, no ductal dilation n Sclerosing cholangitis n Early obstruction p Direct bili elevation, ductal dilation n Choledocholithiasis with obstruction n Periampullary malignancy p Pancreatic adenoca p Distal cholangiocarcinoma p Ampullary adenoca p Duodenal adenoca n Klatskin s, CBD, CHD tumor n RHD or LHD tumor with significant liver dysfunction n Other 24

Pattern of Extrahepatic Cholangiocarcinoma Biliary Anatomy Gallbladder cancer 2-9X more common than ductal cholangioca #1 # Prevalence % of extrahepatic cholangioca 8-13% 10-26% #4 14-37% #3 6% #2 30-50% 25 26 Gallbladder Cancer p Present in 1/200 to 1/500 cholecystectomy specimens p High association with gallstones p Preop Dx n Continuous pain n RUQ mass by palpation, mass in GB on US n Porcelain gallbladder p Intraop n Inflammation out of proportion to history n Hepatic invasion p Postoperative n Pathology p Stage 1-2 potentially cured with cholecystectomy n Liver wedge resection and porta lympadenectomy for Stage 2 n Port site recurrences p Stage 3-4 n Incurable n 3-6 mo median survival n Chemo and XRT-unresponsive 27 Gallbladder Cancer Staging and Survival Staging (TNM) Stage Survival % (5 yr) 1 75 Stage T N M 1 1 0 0 2 2 0 0 3 1-3 T1-2,N1 T3,N0-1 0 4a T4 N0-1 0 4b Tany Tany N2 Any 0 1 2 25 3 12 4 1 Most patient present in Stage 3 or 4 28 Hilar Cholangiocarcinoma (Klatskin s Tumor- Case 2) 29 p Dx Extrahepatic Ductal Cholangiocarcinoma n W/U pruritis, jaundice n US, CT, ERCP, EUS and biopsies (brushings) n Visible mass by preop studies is poor prognostic finding as it usually indicates local extension into porta structures rendering the lesion unresectable p Rx n Resection - <10% resectable for cure n ChemoXRT Equivocal benefit n Luminal XRT (Brachytherapy) experimental n Palliative - Wallstent 30

Case 3 p 54 yo female n H/O Hepatitis C n Increased fatigue, RUQ pain and tenderness n Intermittent confusion noted by family n Exam shows jaundice, ascites. n W/U? Case 3 p 54 yo female n H/O Hepatitis C n Increased fatigue, RUQ pain n W/U p LFT s ALT 100, AST 100, Bili 2.8, Albumin 2.5, INR 2.2 p AFP (nonmaternal) 5 (6 mos ago) rising to 2000 31 32 US Normal liver Case 3: US Margin of mass Mass 33 34 CT Normal Liver Case 3: CT Scan Non-contrast Arterial phase Portal vein invasion PV phase 35 36

Case 3 p 54 yo female n H/O Hepatitis C n Increased fatigue, RUQ pain n W/U p LFT s ALT 100, AST 100, Bili 2.8, Albumin 2.5, INR 2.2 p US Cirrhosis liver masses, bilobar p CT Hypervascular, multifocal masses with right PV invasion p AFP (nonmaternal) Case 3: Multifocal HCC in Cirrhosis Lesion Appearance Dx:HCC in Hep C-associated cirrhosis Options for Management? 37 38 5 (6 mos ago) rising to 2000 p p p p p Hepatitis C (HCV): Overview Leading cause of chronic liver dz US (prevalence 1.4% US population) Transmission: IVDA, transfusions, sexual transmission Most common indication for liver transplantation RNA virus, 4M infections in US, 170M worldwide Natural History n 20-35% progress to cirrhosis n 2-5% of cirrhotics develop HCC per year n 50% of HCV carriers +cirrhosis develop HCC n Timing 10 yr to clinical liver dz 21.2 yr to cirrhosis 29 yr to HCC p Cirrhosis n Survival HCV: Cirrhosis p Compensated dz (CPT 5-9, Child s A and B) 90% 5 yr, 80% 10 yr p Uncompensated dz (CPT >9, Child s C) 50% 5 yr Transplant group p Rx: peginterferon alpha and ribavirin eliminates virus in 55% patients 39 40 Hepatocellular Ca (HCC) - 1 Resection: Gold standard for lesion eradication p Incidence: World-wide very common 560,000 new cases/yr n SE Asia, Sub-Saharan Africa 52/100,000 n NA and Europe 2.5/100,000 p Risk factors chronic liver dz Hep B & C, EtOH p Dx at risk individual, mass, AFP, bx p Resectable dz n 40-50% 5 yr dz free survival but only 10-20% are candidates for resection p Recurrence n satellite nodules or multicentric foci in liver p Resection 40-50% 5 yr dz free survival p Only 30% are resectable for cure p Issues: Resectability, hepatic reserve n Resectability pattern/extent of dz p Staging (US, CT, MR, PET) p Consider preop treatment (RFA, PV embolization) p Segmental vs lobar resection, limiting intraop ischemic injury to liver n Hepatic reserve p Child-Pugh score p 3D reconstruction of liver for assessment of % residual volume (35% normal liver for survival) p Clearance studies (ICG or lidocaine) p Portal-hepatic venous gradient n regional LN, lungs, bone, adrenal 41 42

CT Criteria for Hepatic Segmentation Hepatic Vascular Anatomy Hepatic artery 3D reconstruction Portal vein Spiral CT 43 44 Issues in Hepatic Resection: Control of Bleeding p Hepatic blood flow n 20% of CO to liver n 2/3 in PV (1/3 O 2 ), 1/3 in HA (2/3 O 2 ) p Procedural steps n Mapping lesion and vascular anatomy - IOUS n Dissection of porta n Vascular control/isolation p PV, HA to resected area divided p inflow occlusion (Pringle) p Hepatic vein occlusion (outflow occlusion) p Liver isolation: Heaney maneuver n Parenchymal dissection along anatomic lines p Ligasure p Stapler p Clips and ties Liver resection 45 46 Hepatic Anatomy Resection: Gold standard for lesion eradication 47 p Resection 40-50% 5 yr dz free survival p Only 30% are resectable for cure p Issues: Resectability, hepatic reserve n Resectability pattern/extent of dz p Staging (US, CT, MR, PET) p Consider preop treatment (RFA, PV embolization) p Segmental vs lobar resection, limiting intraop ischemic injury to liver n Hepatic reserve p Child-Pugh score p 3D reconstruction of liver for assessment of % residual volume (35% normal liver for survival) p Clearance studies (ICG or lidocaine) p Portal-hepatic venous gradient 48

Score Child-Pugh-Turcott (CPT) Score Bili (mg/ dl) Albumin (gm/dl) PT (sec) Hepatic Encephal Ascites (grade) 1 <2 >3.5 1 4 None None 2 2-3 2.8 3.5 4 6 1 2 Mild 3 >3 < 2.8 >6 3-4 Severe Sum Score Child s Class Survival (%, 1 yr) 5-6 A 80 7 9 B 70 Alternatives to Liver Resection p Transplantation (for HCC, selected pts) p Embolization n Lipiodol n Chemoembolization p Ablation n Radiofrequency energy n Ethanol n Cryotherapy n Acetic Acid p Radiation n Confocal radiation > 9 C 50 49 50 n Yttrium microspheres (Theraspheres TM ) RFA p Indications: n No evidence of extrahepatic tumor n Less than 5 cm in diameter n Not in proximity to portal triad Transplantation for HCC MELD Feb 2002 Implementation p Criteria (highly selected patients): n One HCC nodule up to 5 cm, OR n Up to 3 lesions (all < 3 cm) n No extrahepatic spread, vascular involvement, not a resection candidate p Survival n 83% at 3 yr 51 52 Liver Mass Biopsy + Suspicious: AFP cirrhosis Hepatocellular Carcinoma Extent of Disease -multiphase CT -lipiodol CT -MRI Limited Disease 1 5 cm 3 3 cm ØPV thrombosis Extensive Disease >5 cm or >3 lesions PV thrombosis Metastases 53 Hepatocellular Carcinoma Limited Disease 1 5 cm 3 3 cm ØPV thrombosis Extensive Disease >5 cm or >3 lesions PV thrombosis Metastases Hepatic Reserve Child s A Child s B Child s C Wedge Pressure Gradient Other factor: Platelet count <10 >10 54

Limited Disease Hepatic Reserve Child s A Child s B Child s C Hepatocellular Carcinoma Wedge <10 Pressure Gradient >10 Performance Status Good Poor Transplant candidate yes Tumor -near limit -progressive no Resection Ablation Transplantation Bridging Rx Ablation Chemo TACE Ablation Chemo XRT TACE Supportive Care 55 Hepatocellular Carcinoma Extensive Disease >5 cm or >3 lesions PV thrombosis Metastases Hepatic Reserve Child s A Child s B Child s C Performance Status Wedge <10 Pressure Gradient >10 Resection Ablation Chemo XRT TACE Supportive Care 56 Metastatic Disease to the Liver p Treatable lesions (for cure, or for palliation of hormonal secretory function) n Colorectal metastases n Carcinoid n Islet tumors n Breast ca? p Characteristics n Isolated to liver n Adequate residual parenchyma and liver function CRC: Metastatic Liver Tumors p 15-25% of patients with colorectal cancer (CRC) have liver metastases at time of dx p 20% of CRC patients develop metachronous liver lesions p 25% of CRC patients have isolated liver metastases n 10-25% are resectable for cure n Survival: 25-35% at 5 years 57 58 Benign Hepatic Tumors p Hepatic cysts Focal Nodular Hyperplasia p Biliary cystadenomas p Hemangiomas p Focal nodular hyperplasia p Hepatic adenoma 59 60

Hepatic Adenoma Case 4 p 58 yo WM, former alcoholic n Symptoms of forgetfulness, fatigue, abdominal distension n Exam shows palmar erythemia, gynecomastia, spider angiomata, distended abd with ascites, jaundice, muscle wasting n Evaluation? 61 62 Case 4 p Labs n Hepatitis screen n LFT s, albumin, INR, platelet count n AFP p Films n US portal vein, liver n CT liver, spleen, ascites p Assessment: Hepatic reserve n Cumulative hepatic volume n CPT score CT Scan p Findings n Shrunken liver n Irregular contour n Perigastric prominent vasculature n Splenomegaly p Dx: Advanced cirrhosis and portal hypertension 63 64 Clinical Manifestations of Portal Hypertension Physiology: Critical Functions p Synthesis n Albumin n Clotting factors n Plasma proteins n Cholesterol/bile salts for digestion p Regulation n Aminoacid metabolism p Energy metabolism n Glycogen - gluconeogenesis p Detoxification n Ammonia to urea for excretion n drugs, metabolites (i.e. octopamine) p Excretion n Bile p Immunological n Bacterial filter, regulation immune response to infection 65 66

Clinical Consequences of Portal Hypertension (PH) p Definition n portal vein pressure >10-13 mm Hg (57% sensitivity, 100% specificity) p Natural History n Most (90%) cirrhotics develop PH eventually n Only 20-30% bleed n Most (80%) of bleeding episodes stop spontaneously n Most (50-70%) rebleeding (1-2 yr) n 1 in 3 dies from complications of first bleeding episode n 50% - 5 yr survival with cirrhosis + PV 67 Portal Hypertension: Medical Rx p Supportive measures (stop EtOH) p Medical Rx: Beta blocker p Banding/sclerosis for bleeding esophageal varices 68 Portal Hypertension: Rx p Supportive measures (stop EtOH) p Medical Rx: Beta blocker p Banding/sclerosis for bleeding esophageal varices p Portcaval shunt n TIPSS (transjugular intrahepatic portosystemic shunt) n Portacaval shunt (nonselective) p Liver transplantation for decompensation and/or complications Portal Venous anatomy 69 Survival after Liver Transplant for Cirrhosis and End Stage Liver Failure UNOS 2001 Annual Report Graft Survival Patient Survival # Pts 1 Year % 5 Year % 6577 80 63 6577 88 74 70 Summary p Surgical Hepatobiliary Disorders n Biliary p Gallstone disease p Biliary tumors p (Cystic diseases of biliary tree) n Liver p Liver tumors Primary malignancy Role of Hep C Secondary malignancy Benign tumors p Liver failure Portal hypertension and complications Liver failure assessment and definitive Rx Transplantation 71